oana epi 2014
DESCRIPTION
EpilepsieTRANSCRIPT
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EPILEPSIA COPILULUI SI ADOLESCENTULUISINDROAME EPILEPTICE (2)
OANA TARTA-ARSENE
CLINICA DE NEUROLOGIE PEDIATRICASPITALUL CLINIC DE PSIHIATRIE “AL. OBREGIA’
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Acest curs!!1. 4 sindroame epileptice (dpdv practic);
Varsta; Tip de crize; Investigatii paraclinice; Diagnostic pozitiv;
SEMIOLOGIC; SINDROMOLOGIC;
Tratament; Prognostic;
2. Tratamentul antiepileptic: Criza epileptica; Epilepsiei – medicamentos vs non-medicamentos;
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Manifestare paroxistica ? EPILEPTICA / NON- EPILEPTICA
? EPILEPSIE ≥ 2 crize epileptice, >24h; 1 criza epileptica + MODIFICARI EPILEPTIFORME PE EEG
(risc de recurenta crescut!)
DIAGNOSTIC SEMIOLOGIC = TIPUL DE CRIZA EPILEPTICA; FOCALA SIMPLA / COMPLEXA (cu/fara pierderea constientei) GENERALIZATA (mioclonica, tonica, clonica, tonico-clonica, absenta etc)
EEG-ul ictal! DIAGNOSTICUL SINDROMOLOGIC (semiologic +
Varsta, AHC, ex clinic neurologic & psihic; Imagistic
DE CAUZA STRUCTURALA/NECUNOSCUTA/GENETICA;(SIMPTOMATICA / CRIPTOGENICA / IDIOPATICA)
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Analize paraclinice
CRIZE EPILEPTICE
FUNCTIA:EEG
STRUCTURA:IRM/CT cerebral
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Bebeloi..
1. Varsta: sugar;2. Tip de crize:
crize generalizate: spasme infantile;3. Investigatii paraclinice;
EEG: hipsaritmie (veghe); Imagistic cerebral: N/aN
4. Diagnostic pozitiv; SEMIOLOGIC=tipul de crize→epi generalizata SINDROMOLOGIC= cauza str/genetica/metabolica (Sdr West)
5. Tratament: ACTH/vigabatrin;
6. Prognostic: prost….
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Crize epileptice.. Nocturne, hipnice (de obicei…1 + 2)
CRIZE GENERALIZATE TONICE AXIALE!!
Veghe, mai multe tipuri:1. Rare..
CRIZE GENERALIZATE ATONE;
2. RARE… CRIZE GENERALIZATE MIOCLONICE
3. Rare.. CRIZE FOCALE CU PIERDEREA CONSTIENTEI (‘ABSENTE
ATIPICE’);
4. Rare… CRIZE FOCALE MOTORII DE HEMICORP DREPT CU PIERDEREA
CONSTIENTEI
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1. SINDROMUL LENNOX-GASTAUT
Debut: 1-8 ani, baietii>fetele;
Clinic: crize epileptice polimorfe (tonice axiale hipnice,
atone, mioclonice, absente atipice, crize partiale etc);
Dezvoltare psihomotorie: anterior debutului crizelor normala/incetinita; dupa debutul crizelor regres/retard psihomotor;
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ANALIZE PARACLINICE Functia:
EEG: VEGHE:…
SOMN:…
Veghe: CVU < 3 c/sec, anomalii
multifocale;
SOMN: ritmuri rapide 10 Hz (crize tonice..);
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ANALIZE PARACLINICE
Structura: IMAGISTICA CEREBRALA:
IRM / CT cerebral: Anormala (de ex malf corticale) Normala..
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SINDROM LENNOX-GASTAUT
Tratament: polidrog, farmacorezistent; eventual
dieta cetogenica, chirurgical;
Prognostic: rezervat (intirziere mentala, tulburari
psihice, epilepsie)
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Sindromul Lennox-Gastaut
1. Varsta: 1-8 ani;
2. Tip de crize: crize focale+generalizate: multiple;
3. Investigatii paraclinice; EEG: veghe (CVU<3Hz); somn-ritmuri rapide Imagistic cerebral: N/aN
4. Diagnostic pozitiv; SEMIOLOGIC=tipul de crize→epi generalizataepi generalizata SINDROMOLOGIC= cauza str/genetica/metabolicacauza str/genetica/metabolica etc etc (Sdr
Lennox-Gastaut)5. Tratament:
Combinatie antiepileptice/trat epi rezistente;6. Prognostic:
prost….
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Manifestari hipnice..
Film CRIZE FOCALE SIMPLE HEMIFACIES DREPT..
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2. EPILEPSIA PARTIALA BENIGNA CU VARFURI CENTRO-TEMPORALE
Predispozitie genetica: AHC + pt convulsii febrile / epilepsie;
Debut 3-13 ani, predominant la sexul masculin; Clinic:
crize partiale motorii/senzitive fara pierderea constientei (debut cu parestezii facio-brahiale, contractii tonico-clonice unilaterale ale fetei +/- membrului superior, anartrie, sialoree) mai ales in timpul somnului;
DPM + ex neurologic: normal;
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ANALIZE PARACLINICE Functia:
EEG:VEGHE:
SOMN:
Modificari focale epileptiforme de virfuri lente CT;
Modificari activate de somn!!
Pot fi focare multiple, dar predominant cel controlateralsemnelor clinice!!
E al baietelului filmat???
DA!!
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ANALIZE PARACLINICE
Structura: IMAGISTICA CEREBRALA:
IRM / CT cerebral; Normal;
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EPILEPSIA PARTIALA BENIGNA CU VARFURI CENTRO-TEMPORALE
Trat: Mentinerea in observatie; eventual VPA;
EXISTA CAZURI CU AGRAVARE LA CARBAMAZEPINA!!
Prognostic: excelent;
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Epilepsia partiala benigna cu varfuri centro-temporale
1. Varsta: 3-13 ani;
2. Tip de crize: crize focale;
3. Investigatii paraclinice; EEG: desc focale/multifocale CT, activate de somn Imagistic cerebral: N
4. Diagnostic pozitiv; SEMIOLOGIC=tipul de crize→epi focalaepi focala SINDROMOLOGIC= cauza geneticacauza genetica
5. Tratament: VPA (atentie la CBZ);
6. Prognostic: Excelent…
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Film
Baietel, 6 ani, zilnic, episoade mm/zi neresponsivitate…
CRIZE GENERALIZATE ABSENTE!!
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3. EPILEPSIA ABSENTA A COPILULUI
Mare predispozitie genetica; AHC + pt convulsii febrile / epilepsie;
Debut: 3-12 ani (max 6-7 ani), mai frecventa la sexul feminin;
Clinic: Crize tip absente de toate tipurile; frecventa 100-200/zi;
DPM + ex neurologic: normal;
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ANALIZE PARACLINICE
Functia: EEG:
Veghe + HIPERVENTILATIE:CVU>3 c/sec, sensibilitate la HV;
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ANALIZE PARACLINICE
Structura: IMAGISTICA CEREBRALA:
IRM / CT cerebral; Normal;
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EPILEPSIA ABSENTA A COPILULUI
Trat: Indicate:
Valproat de sodiu (VPA), Etosuccinimida (ESM), Lamotrigina (LTG);
CONTRAINDICATE: Carbamazepina (CBZ), Fenitoina (PHT), Vigabatrin (VGB);
Prognostic: favorabil;
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Epilepsia absenta a copilului
1. Varsta: 3-12 ani;
2. Tip de crize: crize generalizate: absente;
3. Investigatii paraclinice; EEG: desc generalizate CVU>3Hz la HV!! Imagistic cerebral: N
4. Diagnostic pozitiv; SEMIOLOGIC=tipul de crize→epi generalizataepi generalizata SINDROMOLOGIC= cauza geneticacauza genetica (epilepsia absenta a
copilului)5. Tratament:
VPA, ETS, LTG (nu CBZ, PHT)6. Prognostic:
Excelent…
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4. EPILEPSIA GENERALIZATA IDIOPATICA DE ADOLESCENTA
1. ABSENTE JUVENILE; Predominant crize absente;
2. GRAND-MAL DE TREZIRE; Predominant crize tonico-clonice generalizate;
3. EPILEPSIA MIOCLONICA JUVENILA; Predominant crize mioclonice;
POT EXISTA COMBINATII INTRE ACESTEA!
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EPILEPSIA ABSENTA JUVENILA Debut: 10-17 ani; Clinic:
absente mai scurte, mai rare, mai usoare - fata de absentele copilului;
asocierea cu celelalte forme de EGI; Ex neuro-psihic: normal;
EEG: CVU > 3 c/sec; Trat:
VPA, VPA + ESM, farmacodependente;
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Film
Ioana, 17 ani, la trezire.. Tresare!!
CRIZE GENERALIZATE MIOCLONICE
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EPILEPSIA MIOCLONICA JUVENILA(SDR JANZ)
Debut: 12-16 ani (gena pe bratul scurt al cz 6); Clinic:
crize mioclonice bilaterale, izolate sau repetitive, simetrice/asimetrice, fara pierderea constientei, la trezirea din somn;
Localizare: membrele superioare (umeri), rar membrele inferioare;
Precipitate de privare de somn, SLI, menstra; Ex neurologic & psihic: normal;
EEG: CVU sau PVU > 3c/sec, fotosensibilitate; Trat: VPA; LTG, farmacodependente
PVU la SLI!!
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EPILEPSII GENERALIZATE IDIOPATICE
EPILEPSIA GRAND MAL DE TREZIRE
Debut: 6-20 ani, mai frecventa la fete; Clinic:
tonico-clonice generalizate la trezire sau in perioadele de relaxare; precipitate de privare de somn, SLI, menstra;
EEG: CVU/PVU generalizate > 3 c/sec, fotosensibilitate; Trat: VPA, LTG, farmacodependente;
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EPILEPSIA GENERALIZATA IDIOPATICA DE ADOLESCENTA(CARACTERISTICI COMUNE)
1. ETIOLOGIE - Transmitere genetica (poligenica);2. CLINIC:
1. Frecventa crescuta la fete;2. Debut la adolescenta (cel mai frecvent 13-16 ani);3. Crizele apar la trezire si in veghe;4. Declansare: deprivare de somn, fotostimulare,
menstra sau fara;5. Aspect de crize generalizate primar;
3. Examen neurologic, intelect: normal;4. Examinari neuroimagistice: normale;5. Raspuns bun la tratament, dar farmacodependente:
VPA, LTG;
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EPILEPSIA GENERALIZATA IDIOPATICA DE ADOLESCENTA(CARACTERISTICI COMUNE)
1. Varsta: ADOLESCENTA;
2. Tip de crize: crize generalizate: absente, mioclonii, TCG (combinatii min 2);
3. Investigatii paraclinice; EEG: desc generalizate CVU, PVU>3Hz la SLI!! Imagistic cerebral: N
4. Diagnostic pozitiv; SEMIOLOGIC=tipul de crize→epi generalizataepi generalizata SINDROMOLOGIC= cauza geneticacauza genetica(epilepsia generalizata idiopatica de adolescenta)
5. Tratament: VPA, LTG (farmacodependent…)
6. Prognostic: Excelent…
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Acest curs!!1. 4 sindroame epileptice (dpdv practic);
1. Varsta;2. Tip de crize;3. Investigatii paraclinice;4. Diagnostic pozitiv;
SEMIOLOGIC; SINDROMOLOGIC;
5. Tratament;6. Prognostic;
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Sindroame epileptice
0-1 AN 1-7 ANI 3-13 ANI 13-16 ANI
1. SDR WEST
2. SDRLENNOX-GASTAUT
3. EPILEPSIA PARTIALA BENIGNA
CU VARFURICENTRO-TEMPORALE
4. EPILEPSIAABSENTA ACOPILULUI
5. EPILEPSIAGENERALIZATAIDIOPATICA DEADOLESCENTA
(absenta/mioclonica juvenilaGrand-mal de trezire)
Spasmeinfantile
Mm crize(tonice axiale
Hipnice) Crize focalesimple hipnice
Absente AbsenteMioclonii
Crize TCG
Diagnosticul semiologic!!Diagnosticul semiologic!!
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Sindroame epileptice
0-1 AN 1-7 ANI 3-13 ANI 13-16 ANI
1. SDR WEST
2. SDRLENNOX-GASTAUT
3. EPILEPSIA PARTIALA BENIGNA
CU VARFURICENTRO-TEMPORALE
4. EPILEPSIAABSENTA ACOPILULUI
5. EPILEPSIAGENERALIZATAIDIOPATICA DEADOLESCENTA
(absenta/mioclonica juvenilaGrand-mal de trezire)
HVEx neurologic
normalRETARD PMDEPRIVARE SOMN+ SLI
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Acest curs!!
2. Tratamentul antiepileptic:
Criza epileptica; Epilepsiei – medicamentos vs non-medicamentos;
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TRATAMENT
1. SFATURI ASUPRA CONDITIILOR DE VIATA;2. MEDICAMENTE ANTIEPILEPTICE;3. TRATAMENT CHIRURGICAL;4. DIETA CETOGENICA;5. STIMULARE VAGALA;
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1. CONDITII DE VIATA
Program de somn: regulat; Alimentatia:
interzis consumul de alcool, evitarea excesului de bauturi cofeinizante;
TV, computer, discotecile: interzise la cei cu fotosensibilitate (se recomanda o distanta de minimum 2 metri si ochelari de soare);
Sportul: sunt interzise sporturile care in timpul crizelor pot pune viata pacientilor in pericol (ex. inot)
Vaccinarile: contraindicate antivariolic si antipertusis; Scolarizare: in functie de capacitatea intelectuala;
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2. Medicamente antiepileptice:
Tratamentul crizei epileptice: Pozitie de siguranta; Benzodiazepine:
Diazepam intrarectal 0,5 mg/kgc/doza; Intravenos 0,2 mg/kgc/doza;
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2. Medicamente antiepileptice:
Principii de tratament: Diagnostic cert de epilepsie Individualizare tratament Monoterapie versus politerapie Mod administrare Mentinere
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2. Medicamente antiepileptice:
In functie de semiologia crizelor: Crize epileptice focale:
I-a linie - CARBAMAZEPINA /OXCARBAZEPINA; 20-40 mg/kgc/zi;
Crize epileptice generalizate: I-a linie – VALPROAT DE SODIU;
20-40 mg/kg/zi;
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2. Medicamente antiepileptice:
Alte MAE: Vechi – fenobarbital, fenitoina,
etosuccinimida; Noi – lamotrigina, topiramat, vigabatrin;
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2. Medicamente antiepileptice:
Indicate in: Absente – etosuccinimida;
Contraindicate: Absente – carbamazepina; Mioclonii – carbamazepina,
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2. Medicamente antiepileptice:
Reguli de administrare: 1-ul MAE (doza mica – progresiv doza terapeutica)
↓NERESPONSIV
↓ Al 2-lea MAE (doza mica – progresiv doza terapeutica)
↓NERESPONSIV
↓ Combinatii de MAE
TIMP DE 2-5 ANI / TOATA VIATA!! (personalizat)
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3. Tratamentul chirurgical
Chirurgia epilepsiei CURATIVA = Excizia zonelor epileptogene;
supresia focarului epileptogenic printr-o procedura resectiva;
PALEATIVA; Functionala (reducerea intensitatii si
frecventei crizelor – ex. calosotomia / sectiuni subpiale multiple)
Evaluare prechirurgicala!!;
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Chirurgia epilepsiei curativa
Maria, 5 ani.. Crize epileptice de la vs de 4 ani,
rezistente la tratament..
EEG (interictal)…
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EEG ictal
Aspectul clinic&EEG e concordant cu leziunea..
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Chirurgia epilepsiei PALEATIVA
Calosotomia; Crizele atone / crize focale cu generalizare
secundara!!
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Chirurgia epilepsiei PALEATIVA
Sectiuni multiple trans-subpiale!!
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4. Dieta cetogena Epilepsiile refractare la MAE; Dieta cu continut crescut de lipide + scazut de
glucide si proteine (2 / 3 / 4:1)
Corpi cetonici cantitate crescuta – creste energia cerebrala= stabilitate neuronala (?);
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5. Stimularea vagala Epilepsiile rezistente la tratament Elibereaza un stimul la o perioada fixa:
moduleaza influxul nervos la nivelul subst reticulate ascendente & talamus…:
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Diagnostic diferential tulburari de somn atacuri de panica migrena sincopa tulburari de miscare AIT crize conversive
MANIFESTARI PAROXISTICE NEEPILEPTICE!!
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Epilepsia la oameni celebrii..